Respiratory medicine
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Respiratory medicine · Jul 2010
Therapeutic options for systemic sclerosis related interstitial lung diseases.
Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) mainly encountered in patients with diffuse disease. Unlike idiopathic interstitial pneumonias (IIP), SSc associated ILD corresponds to non-specific interstitial pneumonia (NSIP) in most cases, whereas usual interstitial pneumonia (UIP) is encountered less frequently. This explains the better prognosis of SSc associated ILD compared to IIP. ⋯ Anti-fibrosing treatments have failed to demonstrate any benefit and cyclophosphamide, which has been used in the treatment of this condition for about 15 years, has recently been evaluated in two prospective randomised studies which showed a significant but modest effect on respiratory function. Since none of the patients included in retrospective or prospective studies were selected on the basis of progression of ILD, and since only a minority of SSc patients develop severe ILD, further studies should focus on the subgroup of SSc patients with worsening ILD. A subgroup of patients with rapidly progressive ILD might benefit from pulsed intravenous cyclophosphamide combined with prednisone 15 mg daily but this remains to be confirmed.
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Respiratory medicine · Jul 2010
Bronchoalveolar lavage in idiopathic pulmonary fibrosis: what does it tell us?
Bronchoalveolar lavage (BAL) has only a limited role in diagnosis of idiopathic pulmonary fibrosis (IPF). A finding of raised neutrophils (>5%) and eosinophils (>2%) is characteristic but not diagnostic of IPF. ⋯ In such cases, it may be diagnostic. Because of few and conflicting results BAL fluid analysis has very little clinical relevance determining prognosis and response to treatment in IPF.
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The diagnosis of idiopathic pulmonary fibrosis (IPF) currently requires an integrated clinical-radiological-pathological approach in which the histology plays a different role from in the past. The first reason for this change is that non-invasive diagnostic procedures, particularly pulmonary function tests and high resolution computed tomography, have become increasingly competitive with biopsy in providing prognostic information. The other reason is a better appreciation of the limitations of histology: sampling error and interobserver variation. In this review we analyze the reasons for this change of perspective, provide an update on the practical role of histology in the diagnosis of IPF and discuss some of its complications.
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Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality in adults. Although FEV(1) remains the most important physiologic indicator of the severity of airflow obstruction in COPD, its predictive value for mortality is weak when it is higher than 50% of predicted. ⋯ The validated multidimensional BMI (B), degree of airflow obstruction as expressed by the FEV(1) (O), dyspnea with the modified medical research council (D), and exercise (E) measured with the 6min walk or BODE index encompasses the predictive validity of the best of these variables into a single surrogate measure of disease severity and survival. This article reviews these predictors of mortality in COPD.